Abstract: A gas turbomachine combustion chamber includes bridges extending side by side to connect in one piece a radially inner wall and a radially outer wall towards a free end of the radially inner wall. The bridges, inner wall, and outer wall have an additive layer structure.

Abstract: Disclosed herein are products, methods, and uses for treating, ameliorating, udaying the progression of, and/or preventing a muscular dystrophy or a cancer including, but not limited to, facioscapulohumeral muscular dystrophy (FSHD) or a sarcoma. More particularly, disclosed herein are RNA interference-based products, methods, and uses for inhibiting or downregulating the expression of double homeobox 4 (DUX4). Even more particularly, the disclosure provides nucleic acids comprising U7 DUX4 antisense sequences for inhibiting or downregulating the expression of DUX4 and methods of using said antisense sequences to inhibit or downregulate DUX4 expression in cells and/or in cells of a subject having a muscular dystrophy or a cancer including, but not limited to, FSHD or a cancer.

Abstract: The disclosure provides gene therapy vectors, such as adeno-associated virus (AAV), designed for treatment of an Interferon regulatory factor 2 binding protein like (IRF2BPL) disorder.

Abstract: The disclosure relates to the field of gene therapy for the treatment of a muscular dystrophy including, but not limited to, Duchenne Muscular Dystrophy (DMD). More particularly, the disclosure provides nucleic acids, including nucleic acids encoding U7-based small nuclear ribonucleic acids (RNAs) (snRNAs), U7-based snRNAs, and recombinant adeno-associated virus (rAAV) comprising the nucleic acid molecules to deliver nucleic acids encoding U7-based snRNAs to induce exon-skipping for use in treating a muscular dystrophy including, but not limited to, DMD, resulting from a mutation amenable to skipping exon 44 of the DMD gene (DMD exon 44) including, but not limited to, any mutation involving, surrounding, or affecting DMD exon 44.

Abstract: The present invention relates to methods for shifting the splicing profile of the DUX4 gene, a double homeobox gene on human chromosome 4q35. Recombinant adeno-associated viruses of the invention deliver DNAs encoding U7-based small nucle-ar RNAs to induce DUX4 exon- skipping and the expression of shortened forms of DUX4. The methods have application in the treatment of muscular dystrophies such as facioscapulohumeral muscular dystrophy.

Abstract: The present invention relates to the delivery of oligomers for treating patients with a 5? mutation in their DMD gene other than a DMD exon 2 duplication. The invention provides methods and materials for activating an internal ribosome entry site in exon 5 of the DMD gene resulting in translation of a functional truncated isoform of dystrophin. The methods and materials can be used for the treatment of muscular dystrophies arising from 5? mutations in the DMD gene such as Duchenne Muscular Dystrophy or Becker Muscular Dystrophy.

Abstract: The present disclosure relates to RNA interference-based methods for inhibiting the expression of the dystrophia myotonia protein kinase (DMPK) gene. Recombinant adeno-associated viruses of the disclosure deliver DNAs encoding inhibitory RNAs that knock down the expression of DMPK or interfere with the expression of the CTG repeat associated with myotonic dystrophy type-1 (DM1). The methods have application in the treatment of myotonic dystrophies, including DM1, and other disorders associated with aberrant DMPK expression.

Abstract: A detecting assembly and method for identifying and tracking clouds in a zone of the sky being observed where some thermal-infrared flux emitted by the zone is collected and transmitted to a thermal-infrared detector, the detector including a sensor sensitive to the flux in a set band of wavelengths, a measurement of the actual temperature and actual relative humidity of the air at ground level is carried out and the vertical temperature and water vapor distribution is deduced therefrom, the dataset relating to the thermal-infrared signal emitted by a reference sky for the vertical temperature and water vapor distribution is stimulated or obtained, the dataset thus simulated or obtained is subtracted from the dataset measured by the sensor to determine if clouds are present in the zone, and the dataset thus obtained is processed in order to compute the optical thickness and/or altitude of each cloud in the observation area.

Abstract: A detecting assembly and method for identifying and tracking clouds in a zone of the sky being observed where some thermal-infrared flux emitted the zone is collected and transmitted to a thermal-infrared detector, the detector including a sensor sensitive to the flux in a set band of wavelengths, a measurement of the actual temperature and actual relative humidity of the air at ground level is carried out and the vertical temperature and water vapor distribution is deduced therefrom, the dataset relating to the thermal-infrared signal emitted by a reference sky for the vertical temperature and water vapor distribution is stimulated or obtained, the dataset thus simulated or obtained is subtracted from the dataset measured by the sensor to determine if clouds are present in the zone, and the dataset thus obtained is processed in order to compute the optical thickness and/or altitude of each cloud in the observation area.

Abstract: A method for successive printing of a first print job and a second print job on the same web using at least one printing group of the rotary press to print the first print job and the second print job is provided. The printing group includes at least a plate cylinder, a plate, a blanket cylinder and a blanket. Between the first print job and the second print job, a plate-changing stage occurs in which the blanket is held away from the web and the plates of the plate cylinder are changed. According to one aspect of the invention, between the first print job and the second print job, the web is kept traveling through the rotary press.

Abstract: A system and methods of managing entries in a cuckoo hash table that is subject to manipulation by a plurality of concurrent processors are provided. The cuckoo hash table is used to maintain a plurality of keys, along with their data items, in a plurality of entries. In accordance with an operating method, a first command for the cuckoo hash table is processed, and the first command is associated with a first processor of the plurality of concurrent processors. In addition, invalid conditions associated with concurrent processing of a second command for the cuckoo hash table are prevented or minimized. The second command is associated with a second processor of the plurality of concurrent processors. As one example, the operating method manages the situation where concurrent push commands create a race condition. As another example, the operating method manages the situation where a lookup or delete command from one processor competes with a push command from another processor.

Abstract: A method for successive printing of a first print job and a second print job on the same web using at least one printing group of the rotary press to print the first print job and the second print job is provided. The printing group includes at least a plate cylinder, a plate, a blanket cylinder and a blanket. Between the first print job and the second print job, a plate-changing stage occurs in which the blanket is held away from the web and the plates of the plate cylinder are changed. According to one aspect of the invention, between the first print job and the second print job, the web is kept traveling through the rotary press.

Abstract: A system and methods of managing entries in a cuckoo hash table that is subject to manipulation by a plurality of concurrent processors are provided. The cuckoo hash table is used to maintain a plurality of keys, along with their data items, in a plurality of entries. In accordance with an operating method, a first command for the cuckoo hash table is processed, and the first command is associated with a first processor of the plurality of concurrent processors. In addition, invalid conditions associated with concurrent processing of a second command for the cuckoo hash table are prevented or minimized. The second command is associated with a second processor of the plurality of concurrent processors. As one example, the operating method manages the situation where concurrent push commands create a race condition. As another example, the operating method manages the situation where a lookup or delete command from one processor competes with a push command from another processor.